Systemic sclerosis in DRw52-positive silica-exposed males: a case report

The aims of this paper were to describe clinical and laboratory details of 4 siblings, of whom 2 have systemic sclerosis and to describe this family in the context of reported cases of familial systemic sclerosis. The proband and his affected male sibling share the tissue typing antigens: HLA11, B57, DR13, DRw52,- and DQ2,6. Both were concordant for gender, silica exposure, marital status, migration history and antinuclear factor status but discordant for systemic sclerosis subtype, age at disease onset and ENA status. One non-affected sibling had no disease despite concordance for gender, HLA status, silica exposure, marital status, and migration history.